Pathology of Restrictive Lung Disease Flashcards

1
Q

What is the interstitium?

A

The connective tissue space around the airways and vessels and the space between the basement membranes of the alveolar walls

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2
Q

What are the two basement membranes in the alveoli?

A

Alveolar epithelial (pneumocyte)
Interstitial capillary endothelial cell

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3
Q

Describe the relationship between the two basement membranes in the alveoli.

A

Usually in direct contact

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4
Q

What is found in the usual interstitium?

A

Elastic fibres and the occasional cell, like a fibroblast

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5
Q

Discuss the pathology of the interstitium in lung disease.

A

It becomes thickened due to a space between the two basement membranes

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6
Q

Why is the thickening of the interstitium an issue?

A

Makes it more difficult for the diffusion of gases.

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7
Q

What happens to the lungs due to the inflammatory process due to the thickening of the interstitium?

A

They become rigid and stiff

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8
Q

What happens to FEV1 and FVC and the ratio between the two when the elastic tissue in the lungs loses it’s elasticity?

A

Low FEV1 & Low FVC but FEV1/FVC normal ratio

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9
Q

What may happen to perfusion/ventilation when the small airways are affected by pathology?

A

There is an imbalance between perfusion and ventilation

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10
Q

Describe what a patient may present with if they have a restrictive lung disease.

A

Dyspnoea- SOB on exertion (would have to abnormal for that person).

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11
Q

As the disease progresses, what may happen to the symptoms of a patient w restrictive lung disease?

A

SOB on exertion will g to SOB at rest

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12
Q

What may patients with restrictive lung disease develop?

A

Type 1 respiratory failure
Heart failure

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13
Q

What is the difference between type 1 and 2 respiratory failure?

A

Type 1 respiratory failure- the respiratory system cannot adequately provide oxygen to the body.
Type 2 respiratory failure-respiratory system cannot sufficiently remove carbon dioxide from the body.

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14
Q

What can type 1 respiratory failure lead to?

A

Hypoxemia

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15
Q

What can type 2 respiratory failure lead to?

A

Hypercapnia -> too much CO2 in the bloodstream

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16
Q

What does a CXR of someone with interstitial lung disease show?

A

Reduced lung volumes and increased lung markings

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17
Q

What can a chronic inflammatory response involve the formation of?

A

Granulomas

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18
Q

What can diffuse alveolar damage be associated with?

A

-Major trauma
-Chemical injury / toxic inhalation
-Circulatory shock
-Drugs
-Infection incl viruses
-Auto(immune) disease
-Radiation

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19
Q

Name some of the viruses which can cause death due to DADS (diffuse alveolar damage syndrome).

A

Influenza
SARS Cov1 aka covid
SARS Cov2 aka covid

20
Q

What are most people with DADS going to end up with?

A

Type 1 respiratory failure- often life threatening

21
Q

What happens to cells in DADS to try and attempt to repair the fluid filled alveoli?

A

Proliferation of type 2 pneumocytes
Proliferation of fibroblasts

22
Q

What happens to the lungs as result of DADS?

A

Scarred, solid lungs

23
Q

Name two types of chronic granulomatous response.

A

Sarcoidosis
Hypersensitivity pneumonitis

24
Q

What is sarcoidosis?

A

A multisystem granulomatous disorder

25
Q

What causes sarcoidosis?

A

Uknown

26
Q

Which parts of the body are very likely to be affected by sarcoidosis?

A

Lymph nodes
Lungs

27
Q

How may sarcoidosis present in young adults?

A

Acute arthralgia- joint pain
Erythema nodosum-swollen fat under skin causing bumps
Bilateral hilar lymphadenopathy

28
Q

How else can sarcoidosis, and a number of other respiratory conditions, be diagnosed?

A

Accidently on a CXR

29
Q

Define non-caseating

A

No necrosis in the middle of a granuloma

30
Q

What might you see in lymph node sarcoidosis?

A

Non-caseating epithelioid granulomas

31
Q

Name some of the antigens which may trigger hypersensitive pneumonitis.

A

Thermophilic actinomycetes- mould grown on hay
Bird / Animal proteins
Fungi
Chemicals
Others

32
Q

Describe some of the presenting symptoms in those with hypersensitive pneumonitis.

A

Fever, dry cough, myalgia,
Chills 4-9 hours after Ag exposure

33
Q

What might you hear upon auscultation of someone with hypersensitivity pneumonitis?

A

Crackles, tachyopnoea, wheeze

tachyopnoea= rapid and shallow breathing btw, need to try and remember this

34
Q

Describe the chronic presentation of hypersensitive pneumonitis.

A

Insidious- slowly, no symptoms at first
Malaise, SOB, cough

35
Q

Which two hypersensitivity reactions are there in hypersensitivity pneumonitis?

A

Type III and Type IV

36
Q

What forms in type IV hypersensitivity reactions?

A

Granulomas

37
Q

Which cells are involved in type III hypersensitivity reactions?

A

Lymphocytes

38
Q

Which part of the lungs does hypersensitivity pneumonitis usually affect?

A

Upper zone of the lung

39
Q

When might you see someone develop UIP (Usual Interstitial Pneumonitis)?

A

-If they have a connective tissue disease
-Drug reaction
-Post infection
-Industrial exposure e.g. asbestos

40
Q

What type of hypersensitivity reaction is associated with UIP?

A

Type II

41
Q

What is UIP defined by?

A

Patchy interstitial chronic inflammation
Smooth muscle and vascular proliferation
Proliferating Fibroblastic Foci

-?don’t really need to be concerned about this, more worried about the concept of UIP as a whole

42
Q

List some of the presenting symptoms of Idiopathic Pulmonary Fibrosis (UIP).

A

Dyspnoea, Cough, Basal Crackles, Cyanosis, Clubbing

43
Q

Is UIP progressive or treatable?

A

Progressive
Most die within five years

44
Q

What does the term honeycombing refer to?

A

Fibrosis or End-Stage Honeycomb Lung
i.e. non functional, shrunken, hardened lung

45
Q

Just for a recap question to finish- describe what happens in shunt.

A

Blood passes from the right to left side of the heart without contacting ventilated alveoli